Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model

Friedreich ataxia (FRDA) is an inherited neurodegenerative disorder caused by GAA repeat expansion within the FXN gene, leading to epigenetic changes and heterochromatin-mediated gene silencing that result in a frataxin protein deficit. Histone deacetylase (HDAC) inhibitors, including pimelic o-amin...

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Autores principales: Sandi, Chiranjeevi, Pinto, Ricardo Mouro, Al-Mahdawi, Sahar, Ezzatizadeh, Vahid, Barnes, Glenn, Jones, Steve, Rusche, James R., Gottesfeld, Joel M., Pook, Mark A.
Formato: Artigo
Lenguaje:Inglês
Publicado: Academic Press 2011
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Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3107941/
https://ncbi.nlm.nih.gov/pubmed/21397024
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2011.02.016
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